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Öğe Çocuklarda idrar örneklerinden saptanan toplum kaynaklı gram negatif mikroorganizmaların dağılımı ve 2003-2010 yılları arasında antibiyotik direncindeki artışın değerlendirilmesi(2012) Güner, Şükrü Nail; Göktürk, Bahar; Bayrakçı, Umut Selda; Baskın, EsraAmaç: İdrar yolu enfeksiyonu tedavisinde uygun antibiyotik kullanımı, gelecekte oluşabilecek antibiyotik direncine ve böbrekte parankimal hasar, reflü nefropatisi gibi komplikasyonlara karşı koruma sağlayacaktır. Burada, Konya bölgesinde, toplumdan kazanılmış idrar yolu enfeksiyonu etkenlerini ve zaman içindeki antibiyotik direnci oranlarındaki değişimi göstermek amaçlanmıştır. Gereç ve Yöntem: Temmuz 2003-Ocak 2010 döneminde idrar kültüründe üreme belirlenen 0-17 yaş aralığındaki hastalar araştırmaya alındı. Toplam 1742 hastadan 2544 kültür üremesi elde edildi. Bulgular: Üreme olan hastaların %57,6’sını kızlar oluşturmaktaydı. Kız ve erkek hastalarda enfeksiyon etkeni olarak sırasıyla E.coli %76,1-41,9, Klebsiella spp %13,7–24,3, Proteus spp %6,9-28,4 ve diğerleri %3,4-5,2 oranında saptandı. E.coli, Klebsiella ve Proteus türlerinin en duyarlı olduğu antibiyotikler karbapenemler, aminoglikozitler, kinolonlar ve üçüncü kuşak sefalosporinler olarak belirlendi. E.coli ve Proteus gruplarında yüksek trimetoprim-sulfametoksazol (TMP-SM) direnci (%48,2-48,5), E.coli ve Klebsiella gruplarında ise yüksek ampisilin direnci (%68,9-88,2) dikkat çekiciydi. Her üç mikroorganizma için %100’e yakın amikasin duyarlılığı saptandı. 2003-2006 ve 2006-2010 yılları arasında antibiyotik duyarlılıkları karşılaştırıldığında, mikroorganizmaların birçok antibiyotiğe karşı direnç oranlarının anlamlı düzeyde arttığı görüldü. Çıkarımlar: İdrar yolu enfeksiyonuna neden olan mikroorganizmaların aminoglikozit ve üçüncü kuşak sefalosporinlere duyarlı olduğu görülmüştür. İdrar yolu enfeksiyonu tedavisinde yüksek direnç gelişimi nedeniyle TMP-SM ve ampisilin etkeni bilinmeden yapılan tedavide ilk seçenek olmamalıdır. (Türk Ped Arş 2012; 47: 107-13)Öğe Efficacy of intravenous immunoglobulin treatment in immunocompromised children with H1N1 influenza: a clinical observation(WILEY-BLACKWELL, 2016) Göktürk, Bahar; Pekcan, Sevgi; Güner, Şükrü Nail; Artaç, Hasibe; Keleş, Sevgi; Kıraç, Mine; Reisli, İsmailBackground and AimsThe appropriate treatment of pandemic H1N1 influenza which was first identified in April 2009 in Mexico is insufficient especially for immunocompromised patients. We aimed to evaluate the features and prognostic factors of the children with H1N1, especially immunocompromised ones, and whether intravenous immunoglobulin G (IVIG) replacement could aid for a better outcome. MethodsTwenty-one hospitalized children with laboratory-confirmed H1N1 were evaluated retrospectively. Data were extracted from files and electronic medical records. ResultsThe median age was 37 (1-216) months; 62% of them were under 5years of age and 71.4% had one or more underlying disorders. Main symptoms were high fever, cough, fatigue and vomiting. Lower respiratory tract manifestations were seen in 66.6% of children. Mortality rate was 4.7%. The patient who died had the lowest lymphocyte (100/mm(3)), thrombocyte (21000/mm(3)) and highest blood urea nitrogen (87mg/dL) levels. Fifty-eight percent of evaluated patients had one of the primary immunodeficiency disorders. Surprisingly, none of the six patients with primary immunodeficiency who are on regular IVIG replacement needed intensive care unit and died. Although median durations of cough, fever and hospitalization were lower, they did not change statistically according to get IVIG replacement regularly (P=0.47, 0.97, 0.09, respectively). ConclusionOur study is important while it is the first one that shows the course of primary immunodeficient children with H1N1 infection who were on regular IVIG replacement. A trial of high-dose IVIG may be a useful adjunctive therapy in severe H1N1 influenza, particularly in the immunocompromised patients.Öğe İmmünglobulin A eksikliğinde atopi ve allerjik hastalıklar/Atopy and allergic diseases in immunoglobulin A deficiency(2012) Artaç, Hasibe; Keleş, Sevgi; Karagöl, Cüneyt; Göktürk, Bahar; Reisli, İsmailGiriş: İmmünglobulin (Ig) A eksikliği özellikle okul öncesi çocukluk çağında morbiditeye neden olan yaygın bir immün sistem bozukluğudur. Bu çalışmanın amacı, IgA eksikliği olan olguları atopi ve allerjik hastalık yönünden yaş gruplarına göre değerlendirmek, bu eksikliğin dört yaş altında ve üstündeki klinik sonuçlarını belirlemektir. Gereç ve Yöntem: Kasım 2001-2007 tarihleri arasında 272 parsiyel ve 23 selektif IgA eksikliği olan toplam 295 hasta retrospektif olarak incelendi. Bulgular: Hastaların tanı yaşları 64.3 43 (4-204) ay, infeksiyonların başlangıç yaşı 29.5 36 (1-168) ay idi. Tanı yaşı dört yaş altında 121 hasta mevcuttu. Başvuru sebepleri sırasıyla tekrarlayan bronşit (%40), tonsillofarenjit (%33), sinüzit (%6), pnömoni (%4), otit (%2), akut gastroenterit (%0.3) ve boyun apsesi (%0.3) olarak kaydedildi. Olguların %25’inde eozinofili ve %38’inde artmış IgE düzeyi ve aralarında ilişki belirlendi. Hastaların %31’inde spesifik IgE ve/veya deri testi pozitifliğiyle atopi saptandı. Allerjik hastalık sıklığı %78 oranında bulundu (%60 astım, %15 allerjik rinit, %8 atopik dermatit, %6 ürtiker, %0.7 allerjik konjunktivit). Klinik izlemde IgA düzeyinin normal sınırlara ulaşma zamanı 17 14 ay olup, 3-60 ay arasında değişmekteydi. Selektif IgA eksikliği olan üç hastada bronşektazi mevcuttu. Sonuç: Serum IgA düzeyi yalnızca tekrarlayan infeksiyon durumlarında değil, allerjik semptomlarla başvuran hastalarda da değerlendirilmelidir.Öğe Konjenital Lober Amfizemi Taklit Eden Yabancı Cisim Aspirasyonu(2012) Pekcan, Sevgi; Uygun, Hasibe; Göktürk, Bahar; Altınok, Tamer; Resili, İsmail; Artaç, HasibeÖZET Giriş: Konjenital lober amfizem (KLA), süt çocuğunda solunum yetmezliğine yol açan nadir akciğer gelişim malformasyonlarındandır. Hastalar, sıklıkla yaşamın ilk 6 ayında tekrarlayan solunum sıkıntısıyla başvururlar. Yabancı cisim aspirasyonu (YCA), çocukluk çağında morbidite ve mortalitenin önemli bir sebebidir. Tekrarlayan alt solunum yolu infeksiyonu hikayesi olan ve tedaviye cevap vermeyen hastalarda YCA akla gelmelidir. Olgu Sunumu: KLAi taklit eden uzun süreli solunum şikayeti olan ve radyolojik bulguları 11 aydır havalanma farkı şeklinde sebat eden 16 aylık YCA olgusu sunuldu. Tartışma: Yabancı cismin akut ve kronik dönemdeki radyolojik bulgusu her iki akciğer arasında havalanma farkı olabilir ve bu bulgu küçük çocuklarda bazen KLAi düşündürebilir. (Gün cel Pediatri 2012; 10: 70-3)Öğe Konjenital Lober Amfizemi Taklit Eden Yabancı Cisim Aspirasyonu(Galenos Yayincilik,, 2012) Pekcan, Sevgi; Uygun, Hasibe; Göktürk, Bahar; Altınok, Tamer; Artaç, Hasibe; Reisli, İsmailIntroduction: Congenital lobar emphysema (CLE) is a rare malformation of lung development which may be the cause of respiratory insufficiency of the suckling child. Patients often present within the first 6 months of life with recurrent respiratory distress. Foreign body aspiration (FBA) is an important cause of morbidity and mortality in childhood. Foreign body aspiration should be thought in patients who have a history of recurrent lower respiratory infections and resistance to treatment. Case Report: We report a case of 16 month old girl with FBA mimicking CLE. She was suffering for persistan respiratory symptoms and radiological finding was maintaned as the difference between two sides of the lungs aeration for 11 months. Conclusion: In the acute and chronic period of FBA, the radiological finding can be different between two sides of the lungs aeration and this finding sometimes might suggest CLE in infancy.Öğe Late-Onset Adenosine Deaminase Deficiency Presenting with Heck's Disease(SPRINGER, 2010) Artaç, Hasibe; Göktürk, Bahar; Bozdemir, Şefika Elmas; Toy, Hatice; Van Der Burg, Mirjam; Santisteban, Ines; Hershfield, Michael; Reisli, İsmailFocal epithelial hyperplasia, also known as Heck's disease, is a rare but distinctive entity of viral etiology with characteristic clinical and histopathological features. It is a benign, asymptomatic disease of the oral mucosa caused by human papilloma viruses (HPV). Previous studies postulated an association between these lesions and immunodeficiency. Genetic deficiency of adenosine deaminase (ADA) results in varying degrees of immunodeficiency, including neonatal onset severe combined immunodeficiency (ADA-SCID), and milder, later onset immunodeficiency. We report a 12-year-old girl with the late onset-ADA deficiency presenting with Heck's disease. Our case report should draw attention to the possibility of immunodeficiency in patients with HPV-induced focal epithelial hyperplasia.Öğe OCULAR FINDINGS IN 22Q11.2 DELETION SYNDROME(SPRINGER/PLENUM PUBLISHERS, 2014) Göktürk, Bahar; Bozkurt, Banu; Yıldırım, Mahmut Selman; Reisli, İsmail[Abstract not Available]Öğe Ocular Findings in Children With 22q11.2 Deletion Syndrome(SLACK INC, 2016) Göktürk, Bahar; Yılmaz, Pınar Topçu; Bozkurt, Banu; Yıldırım, Mahmut Selman; Güner, Şükrü Nail; Sayar, Esra Hazar; Reisli, İsmailPurpose: To identify the ocular features of children diagnosed as having 22q11.2 deletion syndrome in a Turkish population, which is the most common microdeletion syndrome with a wide range of facial and ocular abnormalities. Methods: Sixteen children aged between 4 months and 18 years with a microdeletion in chromosome 22q11.2 underwent a detailed ophthalmological examination including uncorrected and best corrected visual acuity testing, stereoscopic vision examination, biomicroscopic and indirect fundus examination, and ocular motility testing. Results: All patients had at least one ocular abnormality. The major abnormalities were eyelid abnormalities (eye hooding, narrow palpebral fissure, telecanthus, hypertelorism, sparse and thin eyebrows and eyelashes, blepharitis, and distichiasis), posterior embryotoxon, and tortuous retinal vessels in at least half of the patients. Other ophthalmological disorders were refractive errors, iris remnants, and strabismus. Conclusions: The chromosome 22q11.2 deletion syndrome is associated with a wide range of ocular disorders, which necessitates a comprehensive eye examination for appropriate treatment and follow-up. Ocular findings sometimes can provide a clue to the diagnosis of 22q11.2 deletion.Öğe Propranolol usage in subglottic hemangioma: Case report(2011) Pekcan, Sevgi; Köksal, Y.; Altıntepe, F.; Aslan, Ebubekir; Göktürk, Bahar; Sakarya, Mehmet Emin; Ödev, KemalInfantile hemangiomas are the most common tumors of infancy. Majority of them occur in the head and neck region and rarely in the respiratory tract. Oral corticosteroids are first-line drugs for such troublesome hemangiomas; interferon alfa and vincristine are used less often because of their toxic effects. Recent literature has described propranolol treatment for the vascular tumors. We present a 20-month-old girl with subglottic hemangioma whose respiratory symptoms did not resolve completely with corticosteroid treatment despite accompanying side effects and who was succesfully treated with oral propranolol. No side effects of propranolol was observed. As a conclusion, we propose that propranolol is a safe and effective treatment which markedly shortens the disease course and which should now be used as a first-line treatment in infantile subglottic hemangiomas. Copyright © 2011 by Türkiye Klinikleri.Öğe Reduced CD19 expression and decreased memory B cell numbers in transient hypogammaglobulinemia of infancy(SPRINGER-VERLAG ITALIA SRL, 2013) Artaç, Hasibe; Kara, Reyhan; Göktürk, Bahar; Reisli, İsmailWe aimed to evaluate the role of the CD19 complex in the pathogenesis of transient hypogammaglobulinemia of infancy (THI) and to better characterize the subsets of memory B cells. The study population consisted of 22 male and 14 female patients with a mean age at presentation of 20 +/- A 9.9 months. The CD19 complex and B cell subsets were evaluated by flow cytometry. While the CD19 median fluorescence index (MFI) in patients with THI was significantly lower than controls (122.9 +/- A 66.7 in patients; 184.2 +/- A 39 in controls, p < 0.01), expression of CD21 and CD81 was increased (94.4 +/- A 3, 96.8 +/- A 2.5 % in patients; 91 +/- A 3.9; 94.7 +/- A 3.5 % in controls, p < 0.01 vs. p < 0.05, respectively). The expressions of switched memory B cells and IgM memory B cells were found to be reduced in THI. Considering that the CD19 complex regulates the events following antigen stimulation, the change in CD19 complex detected in THI may be related to insufficiency of antibody production.Öğe Reduced CD19 Expression and Decreased Memory B Cell Numbers in Transient Hypogammaglobulinemia of Infancy(Wiley-Blackwell, 2012) Artaç, Hasibe; Göktürk, Bahar; Kara, Reyhan; Reisli, İsmailWe aimed to evaluate the role of the CD19 complex in the pathogenesis of transient hypogammaglobulinemia of infancy (THI) and to better characterize the subsets of memory B cells. The study population consisted of 22 male and 14 female patients with a mean age at presentation of 20 ± 9.9 months. The CD19 complex and B cell subsets were evaluated by flow cytometry. While the CD19 median fluorescence index (MFI) in patients with THI was significantly lower than controls (122.9 ± 66.7 in patients; 184.2 ± 39 in controls, p\0.01), expression of CD21 and CD81 was increased (94.4 ± 3, 96.8 ± 2.5 % in patients; 91 ± 3.9; 94.7 ± 3.5 % in controls, p\0.01 vs. p\0.05, respectively). The expressions of switched memory B cells and IgM memory B cells were found to be reduced in THI. Considering that the CD19 complex regulates the events following antigen stimulation, the change in CD19 complex detected in THI may be related to insufficiency of antibody production.Öğe Systemic Atopy and Immunoglobulin Deficiency in Turkish Patients with Vernal Keratoconjunctivitis(TAYLOR & FRANCIS INC, 2013) Bozkurt, Banu; Artaç, Hasibe; Arslan, Nasha; Göktürk, Bahar; Bozkurt, Mete Kaan; Reisli, İsmail; Irkec, MuratPurpose: To determine the prevalence of systemic atopy and immunoglobulin (Ig) deficiencies in vernal keratoconjunctivitis (VKC). Methods: Sixty-seven VKC subjects (79.1% boys) with a mean age of 11.3 +/- 4.3 years were included. Serum Ig levels and specific IgE levels were measured using the nephelometric method and reversed enzyme immunoassay with sandwich ELISA technique, respectively. The patients underwent epidermal skin tests with commercial extracts. Results: Family history of atopy and associated systemic allergies were detected in 32.8 and 40.3% of the subjects, respectively. Blood eosinophilia, elevated total, and specific IgE and positive skin tests were detected in 33.8, 42.2, 50, and 35% of the subjects, respectively. Out of 62 subjects, low levels of IgA, IgG, IgM, and IgG3 were detected in 12.9, 8, 6.5, and 1.6% of the patients, respectively. Conclusion: IgE-mediated mechanisms are involved in approximately 40% of VKC patients. A new finding was the higher incidence of Ig deficiency.Öğe Transient hypogammaglobulinemia and unclassified hypogammaglobulinemia: 'Similarities and differences'(WILEY, 2010) Keleş, Sevgi; Artaç, Hasibe; Kara, Reyhan; Göktürk, Bahar; Özen, Ahmet; Reisli, İsmailTransient hypogammaglobulinemia of infancy (THI) is characterized by recurrent infections and one or more reduced serum immunoglobulin (Ig) levels. Usually, this clinical picture resolves spontaneously by 3 yr of age. However, hypogammaglobulinemia persists until adolescence in some patients. In recent years, those patients have been classified as undefined/unclassified hypogammaglobulinemia (UCH). We aimed to evaluate the clinical and immunologic features of patients with THI and UCH considering age of recovery and to assess relationships between hypogammaglobulinemia, infections, and allergic manifestations. We reviewed the medical records of children followed with a diagnosis of hypogammaglobulinemia from 2001 to 2007. Patients with decreased levels (< 2 s.d.) of one or more major Ig isotypes (IgG, IgA, IgM) with normal antibody responses and lymphocyte subpopulations were included (n = 374). Those patients whose Igs normalized during the follow-up period were classified as THI and the others as UCH. The THI group consisted of 71 patients (27 females, 44 males) with a mean recovery age of 68.87 +/- 36.5 months. About 95% of patients with THI recovered before 10 yr of age. The UCH group consisted of 303 patients (105 females, 198 males) with a mean age at diagnosis of 52 +/- 42 months. The most common presenting manifestations in the THI and UCH groups were upper respiratory tract infections (URTIs), lower respiratory tract infections, and asthma (42%, 50%, and 52% in the THI group vs. 39%, 53%, and 55% in the UCH group, respectively). In the THI group, the prevalence of atopic disease was related to age and found to be increased markedly after 44 months. In all patients, the prevalence of asthma was independently and positively associated with family history of atopy and age, whereas it was negatively associated with recurrent URTIs. Patients with THI and UCH have similar clinical and immunologic features. The normalization of Igs may be delayed in a majority of the patients with hypogammaglobulinemia. This observation may be a contribution to the classical definition and diagnostic criteria for THI.Öğe THE VALUE OF MEAN PLATELET VOLUME/PLATELET COUNT RATIO TO PREDICT 22q11.2 DELETION SYNDROME(SPRINGER/PLENUM PUBLISHERS, 2014) Göktürk, Bahar; Güner, Şükrü Nail; Kara, Reyhan; Kıraç, Mine; Keleş, Sevgi; Artaç, Hasibe; Reisli, İsmail[Abstract not Available]Öğe Yaygın değişken immünyetmezliklerde bilinen genetik defektler(2014) Kara, Reyhan; Göktürk, Bahar; Acar, AynurYaygın değişken immünyetmezlik (YDİY), immünyetmezlik hastalıklarının nispeten sık görülen bir şekli olup, immünglobulin üretiminde eksiklik ve primer antikor yetmezliği ile giden heterojen bir hastalık grubudur. Son yıllarda, tanımlanan çeşitli monogenik defektlerin YDİY'in klinik ve laboratuvar bulgularındaki değişkenliği belirlediği ve immünopatogenezinde rol oynadığı anlaşılmıştır. Bu derlemede, YDİY ile ilişkili olan ICOS (inducible co-stimulator), TACI (transmembrane activator and calcium-modulator and cyclophilin ligand interactor), CD19, MSH5 (MutS Homologue 5 Mutations), BAFF-R (B cell activating factor receptor), CD20, CD81, CD21, LRBA (lipopolysaccharide-responsive beige-like anchor) molekül defektleri ve bunların genetik temellerinin gözden geçirilmesi amaçlanmıştır.